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Article

Phenylalanine hydroxylase: function, structure, and regulation.

IUBMB life - 1 Apr 2013

Flydal Marte I, Martinez Aurora

Abstract excerpt

Mammalian phenylalanine hydroxylase (PAH) catalyzes the rate-limiting step in the phenylalanine catabolism, consuming about 75% of the phenylalanine input from the diet and protein catabolism under physiological conditions. In humans, mutations in the PAH gene lead to phenylketonuria (PKU), and most mutations are mainly associated with PAH misfolding and instability. The established treatment for PKU is a...

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Identifiers

PMID: 23457044
DOI: 10.1002/iub.1150

Genes

PAH

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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